Primary Cutaneous Follicular Helper T-Cell Lymphoma: A Clinicopathological Study of Six Cases and Review of the Literature.
Jianlan Xie, Zhu Li, Xiaoge Zhou et al.
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Case in brief
This study presents six cases of primary cutaneous follicular helper T-cell lymphoma (pcTFH-L), characterized by localized skin lesions in patients aged 42 to 75 years, without systemic symptoms. Histopathological examination revealed dermal infiltration by atypical lymphocytes, with a nodular or diffuse pattern. All cases expressed CD4 and multiple TFH markers, with variable clinical outcomes, including stable disease and complete remission, while two patients experienced severe complications.
Diagnostic / clinical pearl
The consistent expression of PD-1, ICOS, and CXCL13 in pcTFH-L is crucial for accurate diagnosis and differentiation from other cutaneous T-cell lymphoproliferative disorders.
Why it matters
Understanding the immunophenotypic profile of pcTFH-L can aid clinicians in diagnosing this rare lymphoma and managing patient outcomes effectively.